Hypercementosis of the teeth, loss of visual acuity | MDS Coaching AIPGDEE preparations B-IDS

Patient present with Cotton Wool appearance of bone, Hypercementosis of the teeth, loss of visual acuity. The Diagnosis is ?
A. Paget's disease
B. Osteogenesis Imperfecta
C. Cleidocranial Dysostosis
D. Fibrous Dysplasia

Ans. (A)

Clinical Feature- Nausea. dizziness, syncope. ataxia. incontinence, and dementia are cerebellar or brainstem compressive syndromes.

Oral Manifestations- Involvement of jaw is common occurrence. there is predilection for maxilla,

Maxilla:Mandible=2.3:1.

Roentgenographic Features- The osteoblastic phase of osteitis deformans is the more commonly recognized one and inevitably occurs regardless of pre-existing osteOlytic le sions. The osteoblastic areas, which appear as opacities in roentgenogram, tends to be patchy in distribution, eventually becoming confluent, but often still showing minute areas of variations in radiodensity. This patchiness has been termed as 'Cotton-Wool' appearance and is especially well demonstrared in the skull and jaws.

The teeth themselves and adjacent bone present Significant roentgenographic changes suggestive of osteitis deformans also. These consists characteristically of a rather pronounced hypercementosis, and often, loss of a well defined lamina dura around the teeth. Root resorption has been reported in some cases, but this IS unusual.